Fibrous dysplasia (FD) is a benign but slowly progressive disorder of bone in which normal cancellous bone is replaced by immature woven bone and fibrous tissue. Fibrous dysplasia is a very uncommon disorder and the total number of cases is not known. It is usually diagnosed in children and young adults. Some patients have only one bone affected (monostotic), whereas other patients have numerous bones affected (polyostotic). Multiple affected bones are often found on one side of the body. The craniofacial involvement causes very difficult therapeutic problem due to localisation and uncontrolled proliferation followed by compression, both resulting in facial asymmetry, pain, cranial nerve deficiencies, alterations in hearing and loss of vision. Treatment of FD depends on the associated symptoms and may include a range of therapies from observation to complete surgical excision. We describe a rare case of polyostotic fibrous dysplasia with craniofacial localisation associated with the involvement of the humerus in a 65-year-old female patient. The first symptoms in the patient were noted 4 years ago. The diagnosis was based on clinical examination, X-ray, CT and histological examination.